Open Label Study of Subcutaneous Immunoglobulin (SCIg) in Myasthenia Gravis

Myasthenia gravis (MG) is a rare autoimmune disorder which causes the muscles to become weak
because the immune system attacks the connection between the nerves and the muscles.

Hizentra is a subcutaneous (under the skin) immunoglobin (SCIg). An immunoglobin is a blood
protein. Hizentra is being studied for the treatment of patients with MG. Hizentra is
administered by an injection into the skin through a portable infusion pump, which may be
easier for patients to administer than the current treatments.

Participants will be asked to complete 9 clinic visits and 3 telephone calls. It could take
up to 30 weeks to complete all study visits.

Inclusion Criteria:

- Must have MGFA MG grades 2, 3, or 4 generalized MG, according to the MGFA
classification system

- Elevated AChR or MuSK Ab

- Patient's signs and symptoms should not be better explained by another disease process

- IVIg maintenance dose of 0.2 to 2 gm/kg/4 weeks or equivalent dose administered every
2-4 weeks

- Stable IVIg for at least 3 cycles

- Able to complete the study and return for follow-up visits

- Able to give written informed consent before participating in the study

Exclusion Criteria:

- History of chronic degenerative, psychiatric, or neurologic disorder other than MG
that can produce weakness or fatigue

- Other major chronic or debilitating illnesses within six months prior to study entry

- Female patients who are premenopausal and are (a) pregnant, (b) breastfeeding, or (c)
not using an effective method of double barrier birth control

- Altered levels of consciousness, dementia, or abnormal mental status

- Thymectomy in the previous three months

- History of renal insufficiency or liver disease

- Skin disease that would interfere with assessment of injection site reaction

- History of severe reactions to IVIg or SCIg

- Participation in a research study within the last 3 months

- Treatment with rituximab or other biologics within 12 months of study entry

- Unable to provide informed consent